Background
Reference number | Author | Age (years) | Site | Size (cm) | Tumour markers | Signs and Symptoms at presentation | Treatment | Sarcomatous overgrowth | Endometriosis | Hormonal therapy | Follow-up |
---|---|---|---|---|---|---|---|---|---|---|---|
4 | Douglas | 18 gravida | Retroperitoneum | 5 × 4.5 × 4 | NR | Anorexia, suprapubic-low back pain, loss of weight, vaginal bleeding, preterm delivery. 24 weeks | CHT (MTX) | NR | No | No | DOD 10 weeks later with distant metastasis |
5a | Clement | 45 | Right Pelvic peritoneum (pelvic mass that extended into the rectum and the bladder) | 7x7x5 | NR | Right lower leg thrombophlebitis, right paravaginal mass | Surgery (partial tumour resection) + RT | NR | No | No | DOD 9 months later due to pelvic recurrence and visceral metastasis |
5b | Clement | 73 | Midline Pelvic peritoneum, displcing the bladder anteriorly | 14 | NR | Large pelvic mass with bilateral hydronephrosis. Inability to void | Surgery (complete tumour resection) | NR | No | No | DOD 2 months later (postoperatively massive gastric bleeding necessitating a subtotal gastrectomy occurred but after that the patient’s conditions deteriorated gradually-autopsy not done-) |
5c | Clement | 58 | Left Pelvic peritoneum | 16x15x8 | NR | Large pelvic mass. Urinary urgency, rectal pressure | Surgery (partial tumour resection) | NR | Yes | No | AWD local recurrence 15 months later (RT), lung metastases 45 months later (resected) |
6 | Bard | 46 | Right pelvic peritonum (the mass was adherent to the right bladder wall and surrounded the right ureter) | 10 × 8 | NR | Weakness and pelvic pain in the right lower extremity. Urinary incontinency | Biopsy + RT | NR | NR | No | DOD 11 weeks later with distant sepsis and metastasis |
7 | Kao | 42 | Left round ligament | 10 | NR | NR | Surgery (partial tumour resection) + CHT (Cyt) + RT | NR | No | No | DOD after 10 months due to the tumor |
8 | Russell | 29 | Left Broad ligament | 7x6x5 | N.R. | Lower abdominal pain for 2 months and occasional dyspareunia | Surgery (tumour resection) | NR | No | No | Recurrence after 5 months treated with surgery (hysterectomy, bilateral salpingo-oophorectomy) + RT; Died of melanoma after 9 years. |
9 | Kerner | 32 gravida | Broad ligament | 15x10x6 and 10x7x4 | NR | Abdominal pain at 28 weeks | Surgery (tumour resection) | No | No | No | AWD omentum and infundibulopelvic ligament recurrence 22 months later |
10 | Vara | 62 | Bladder | – | NR | Haematuria, weight loss, suprapubic pain | Surgery (radical cystectomy + urethrectomy) | NR | Yes | No | FOD 12 months |
11 | Roman | 55 | Retroperitoneal | NR | NR | NR | Surgery (tumour resection) + RT | NR | Yes | No | First abdominal recurrence 3 years later (resection by thoracoabdominal approach + MPA), a 5 cm perihepatic recurrence 5 years later was completely resected, second perihepatic recurrence 7 years from original tumour (resection of recurrence and TMX), 6 months later intrahepatic metastasis (CHT for recurrences (cisplatin-ifosfamide, ifosfamide, doxorubicin), later atrial tumour (resection of cardiac tumour and oral therapy with etoposide). Died after 10 years from original tumour and 70 days after resection of cardiac tumour. |
12 | De Jonge | 16 | Pelvic peritoneum and infracolic omentum | NR | Ca 125 > 190 U/ml | Severe Abdominal distension and pain | Surgery (tumour resection with extirpation of pelvic mass, left fallopian tube, infracolic omentum and appendix) | Yes | No | NR | Three weeks after primary surgery a pelvic mass recurred (CHT with doxorubicin and ifosfamide), first recurrence of the pouch of Douglas 8 months later (CHT cisplatinum, etoposide and ifosfamide), second recurrence of the pouch of Douglas 14 months later the first recurrence (bilateral salpingo-oophorectomy, abdominal hysterectomy, pelvic and para-aortic lymphadenectomy and hormonal therapy) FOD 57 months after the last cycle of chemotherapy |
13 | Benda | 65 | Vaginal apex | 10x7x8 | NR | Pelvic pressure and urinary frequency | Surgery (tumour resection) | NR | No | No | Three years later a 6 cm vaginal recurrence was completely resected; 5 yrs. after first recurrence a 12 cm vaginal recurrence was completely resected and a progesterone therapy was delivered; 7 years after the second recurrence a 17 cm pelvic recurrence was partially resected and a TMX (2 weeks) therapy followed by P (2 weeks) therapy was delivered; 10 months after the third recurrence a fourth pelvic recurrence was partially resected and treated with RT. AWD 16 years |
14 | Ostor | 49 | Pouch of Douglas | 19x8x3 | NR | Right Iliac fossa pain | Surgery (tumour resection and abdominal hysterectomy and bilateral salpingo-oophorectomy) Radiotherapy and Hormonal therapy (Medroxyprogesterone) | NR | No | No | AWD recurrence 5 weeks later (chemotherapy cisplatin and ifosfamide). Persistence of some nodularity on the pelvic floor 18 months later |
15 | Inoue | 54 | Left paracolpium | 15x11x10 | Ca 125: 860 U/ml | Brownish vaginal discharge Small ulcer in the left posterior fornix | RT+ Surgery (tumour resection+ total abdominal hysterectomy, bilateral adnexectomy, abdominal perineal resection with colostomy) | NR | Yes | No | FOD 1 year later |
16 | Judson | 42 | Vaginal cuff | 6 × 3 | NR | Endometriosis recurred three times and was treated with surgery, hormonal therapy (megestrol, danazol) and brachytherapy. Lesion coming out from vagina | CHT (paclitaxel and carboplatin followed by TMX) | NR | Yes | No | 12 months later a 4 cm vagina recurrence was excised and a RT was delivered than was FOD |
17 | N’Senda | 54 | Liver | 20 × 12 | CA 15–3 and CA 19–9, were three and fourfold normal level respectively | Right-sided epigastric pain | Surgery (tumour resection: A segment -IV enlarged right hepatectomy extended to adjacent diaphragm) | No | Yes | HRT | FOD 24 months |
18 | Kato | 20 | Abdominopelvic peritoneum | 23x23x14 | CA 125: 1000 IU/ml | Fatigue and constipation | Surgery (tumour resection) | NR | No | No | FOD 1 years later |
19a | Yantiss | 36 | Sigmoid | 10,5 | NR | Hypermenorrhoea, 6 months abdominal pain | Surgery (tumour and sigmoid resection) | NR | Yes | NR | FOD 36 months |
19b | Yantiss 2000 | 50 | Colon | NR | NR | NR | Surgery (tumour and colon resection) | NR | Yes | NR | FOD 24 months |
19c | Yantiss 2000 | 83 | Small bowel | 15 | NR | Abdominal mass obstruction | Surgery (tumour and bowel resection) | NR | Yes | NR | NR |
19d | Yantiss 2000 | 43 | Small bowel | 6.5 | NR | NR | Surgery (incomplete tumour resection) | NR | NR | ER T | NR |
20 | Visvalingam | 50 | Abdominopelvic peritoneum | 13 kg | NV | Painless abdominal swelling | Surgery (tumour resection), Hormonal therapy (progesterone) | NR | No | No | Ten months later a 50 cm pelvic recurrence was resected (tumour debulking, extrafascial hysterectomy, omentectomy, appendicectomy). DOD 16 months later (Autopsy revealed tumor nodules throughout the abdominal and pelvic cavity limited to the peritoneal surface) |
21 | Anderon | 46 | Vagina | 10 cm | NR | Removal of the vaginal mass, stalk and paravaginal tissue. | No | Yes | Yes | FOD after a parametrium recurrence that was treated with external radiotherapy and interstitial brachytherapy. | |
22 | Dincer | 50 | Perisplenic Peritoneum | NR | NR | Large bowel obstruction in a woman with Endometriosis treated with aromatase inhibitor | Surgery (partial tumour resection) + Chemotherapy (anthracycline) + experimental anti-angiogenesis agent | Yes | Yes | No | DOD 13 months later due to no regression of the pelvic tumour |
23 | Hines | 43 | Peritoneum (from posterior cul-de-sac through the middle to upper abdomen) | NR | Ca 125: 824 IU/ml | Dysmenorrhea and endometriosis | Surgery (tumour resection) + Hormonal therapy (medroxyprogesterone acetate) | No | Yes | No | FOD 10 months later |
24 | Murugasu | 23 | Pouch of Douglas | 11 | Ca 125: 378 IU/ml; CEA: 13. | Right-sided pelvic pain | Surgery (tumour resection), Chemotherapy (Mesna, adriamycin, ifosfamide), Radiotherapy | Yes | Yes | No | FOD 1 years later |
25 | Liu | 56 | Vaginal Vault | 16 | NR | Urinary incontinence and prolapse in a woman with Vaginal endometriosis (TAH, BSO). | Surgery (tumour resection with adherent structures including rectum and part of the bladder wall) + Chemotherapy (ifosfamide and cisplatin) + Radiotherapy | No | Yes | ERT | FOD |
26 | Raffaelli | 50 | Rectovaginal septum | Not reported | NR | Deep dyspareunia, rectal pain, periovulatory pelvic pain in a woman with diagnosis of endometriosis | Surgery (Hysterectomy, left salpingo oophorectomy and partial vaginectomy) + Hormonal therapy (megestrol acetate) | No | Yes | No | Pelvic recurrence 14 months after first surgery (resection of the mass, chemotherapy with ifosfamide and epirubicin-stopped due to intolerance- and radiotherapy 52,90Gy-stopped due to toxic side effect) FOD 9 months after the last surgery |
27 | Toyoshima | 52 | Vaginal cuff | 11 cm | High level of Ca125 | Neoadjuvant therapy and surgical removal of the tumour, the vaginal wall and the greater omentum | Yes | Yes | No | After a month the first lung recurrence treated with chemotherapy, and then a second abdominal recurrence treated with chemotherapy. DOD after 9 months from surgery | |
28 | Kanngurn | 48 | Pelvic peritoneum | 26x26x10 cm | NR | Right lower quadrant pain | Surgery (tumour resection, Hysterectomy, bilateral salpingo oophorectomy) + Chemotherapy (Bleomycin, Etoposide and Cisplatinum × 7 cycles). | Yes | No | No | Abdominal recurrence 8 months later during the fourth cycle of therapy (13, 5 × 7, 8 × 13 cm), lost at follow-up. |
29 | Chang | 37 | cul-de-sac | 3.5 | NR | Vaginal bleeding in a woman with endometriosis (TAH, BSO and hormonal therapy) | Surgery (resection of cul-de-sac tumour, left anterior proctectomy, coloanal anastomosis) | No | yes | yes | FOD 36 months |
30 | Milam | 47 | Right inguinal channel | 12 × 4 | CA 125: 76,8 | Persistent and enlarged groin mass | Surgery (tumour resection) | No | Yes | HRT | FOD 12 months later |
31 | Huang | 41 | Mesentery of the terminal ileum, right colon and pelvic sidewall | 10 | NV | Right lower quadrant pain and nausea | Surgery (TAH, bilateral salpingo oophorectomy, omentectomy, resection of cul-de-sac and sigmoid colon nodules, and pelvic and para-aortic lymph node dissection) + Chemotherapy (ifosfamide+ cisplatin) | No | Yes | No | Peritoneal recurrence at 1 month and chemotherapy (liposomal doxorubicin) FOD for 18 months |
32 | Han | 34 | Vagina | 7 × 6 | High value | Tumour resection, hysterectomy and bilateral salpingo-oophorectomy | No | Yes | No | The patient had 4 vaginal recurrences. The first was treated with chemotherapy, the second was treated with surgery, the third with surgery and adjuvant therapy and the fourth with chemotherapy. FOD | |
33 | Maeda | 47 | Left pelvic side wall | 3 | NR (LDH level 993 IU/ml) | Acute lower abdominal pain due to pedunculated subserosal myoma | Surgery (tumour resection, bilateral salpingo-oophorectomy, total abdominal hysterectomy) | Yes | Yes | TMX | Four weeks later 8 cm recurrent tumour in the right pelvis treated with salvage surgery. One month after second surgery recurrent tumour in pelvis and upper abdomen that was treated with salvage chemotherapy (liposomal doxorubicin), 5 cycles). FOD three months after chemotherapy. |
34 | Patrelli | 49 | Pouch of Douglas | 10 × 6 | CA 125 and Ca 19–9 slightly elevated | Pelvic pain | Surgery (tumour resection) | Yes | No | No | Eighteen months later recurrence of posterior vaginal fornix (resected), 6 months after recurrence another posterior vaginal fornix recurrence (radical hysterectomy with bilateral salpingo-oophorectomy, and pelvic lymphadenectomy and Radiotherapy-50Gy-) FOD |
35 | Clarke | 50 | Pelvic peritoneum (partially adherent to the posterior uterine serosa) | 34x14x7 | NR | NR | Surgery (omentectomy, appendicectomy, removal of small bowel mesenteric implants) | NR | NR | NR | |
36 | Karateke | 26 | Pouch of Douglas | 18 | NR | Lower abdominal distention and left lower quadrant pain | Surgery (tumour resection) | No | Yes | No | FOD 24 months later |
37 | Yang | 36 | Rectum | 2,5 × 2 | NR | Loose stool, dysmenorrhea, deep dyspareunia, haematochezia | Surgery (tumour resection) | No | Yes | No | FOD 60 months |
38 | Kar | 30 | Omentum | NR | NR | Abdominal distension due to abdominal mass and free fluid | Surgery (hysterectomy, bilateral salpingo oophorectomy, omentectomy) and preoperative chemotherapy | Yes | Yes | No | NR |
39 | Pontrelli | 58 | Vagina | 5 cm | NR | Bleeding vaginal lesion | Vaginectomy and parametrectomy using the laparoscopic approach, total colpectomy and partial cystectomy. After that the patient was candidate for progestin therapy. | Yes | Yes | Yes | FOD at November 2016 |
40 | Mandato | 79 | Abdominal Peritoneum | 16 × 11 | NV | Abdominal distension | Surgery (tumour resection, bilateral salpingo-oophorectomy, total hysterectomy) Residual absent | Yes | No | No | 4 weeks later had a pelvic recurrence; DOD 4 months after diagnosis |
Methods
Systematic review of the literature
Statistical analysis
Case presentation
Results
Clinical features
Treatment
Risk factors
Total population | Endometriosis | P value | Overgrowth | P value | Heterologous sarcomatous differentiation | P value | ||||
---|---|---|---|---|---|---|---|---|---|---|
n = 41 n (%) | No n = 16 n (%) | Yes n = 25 n (%) | No n = 32 n (%) | Yes n = 9 n (%) | No n = 37 n (%) | Yes n = 4 n (%) | ||||
Age (mean ± SD), years | 44.5 ± 15.0 | 43.1 ± 15.8 | 45.5 ± 12.7 | 0.597 | 45.4 ± 13.9 | 41.4 ± 14.6 | 0.463 | 46.4 ± 13.0 | 27.5 ± 11.8 | 0.009 |
Size (mean ± SD), mm | 12.2 ± 6.9 | 13.4 ± 8.3 | 11.0 ± 5.3 | 0.319 | 12.5 ± 6.7 | 11.0 ± 8.1 | 0.659 | 11.9 ± 6.8 | 14.7 ± 8.5 | 0.517 |
Site | 0.066 | 1 | 1 | |||||||
Extra-genital | 32 (78.0) | 15 (93.8) | 17 (68.0) | 25 (78.1) | 7 (77.8) | 29 (78.4) | 3 (75.0) | |||
Vagina | 9 (22.0) | 1 (6.2) | 8 (32.0) | 7 (21.9) | 2 (22.2) | 8 (21.6) | 1 (25.0) | |||
Endometriosis | 1 | 0.281 | ||||||||
No | 16 (39.0) | – | – | – | 13(40.6) | 3 (33.3) | 13 (35.1) | 3 (75.0) | ||
Yes | 25 (61.0) | – | – | – | 19 (59.4) | 6 (66.7) | 24 (64.9) | 1 (25.0) | ||
Overgrowth | 1 | 1 | ||||||||
No | 32 (78.0) | 13 (81.3) | 19(76.0) | – | – | 29 (78.4) | 3 (75.0) | |||
Yes | 9 (22.0) | 3 (18.7) | 6 (24.0) | – | – | 8 (21.6) | 1 (25.0) | |||
Treatment | 0.557 | 1 | 0.101 | |||||||
Surgery | 22 (53.7) | 9 (56.3) | 13 (52.0) | 18 (56.3) | 4 (44.4) | 19 (51.4) | 3 (75.0) | |||
Surgery + additional treatments | 16 (39.0) | 5 (31.2) | 11 (44.0) | 11 (34.4) | 5 (55.6) | 16 (43.2) | 0 (0.0) | |||
No Surgery | 3 (7.3) | 2 (12.5) | 1(4.0) | 3 (9.4) | 0 (0.0) | 2 (5.4) | 1 (25.0) | |||
Surgical Approach | 0.345 | 1 | 0.327 | |||||||
Complete resection | 33 (80.5) | 11 (68.8) | 22 (88.0) | 25 (78.1) | 8 (88.9) | 30 (81.1) | 3 (75.0) | |||
Partial resection | 5 (12.2) | 3 (18.7) | 2 (8.0) | 4 (12.5) | 1 (11.1) | 5 (13.5) | 0 (0.0) | |||
No surgery | 3 (7.3) | 2 (12.5) | 1 (4.0) | 3 (9.4) | 0 (0.0) | 2 (5.4) | 1 (25.0) | |||
Lost in follow up | 6 (14.6) | 4 | 2 | 4 | 2 | 6 | 0 | |||
Status at last follow up (35 patients) | 0.002 | 0.843 | 0.278 | |||||||
FOD | 22 (62.9) | 3 (25.0) | 19 (82.6) | 17 (60.7) | 5 (71.4) | 19 (61.3) | 3 (75.0) | |||
AWD | 4 (11.4) | 3 (50.0) | 1 (4.3) | 4 (14.3) | 0 (0.0) | 3 (9.7) | 1 (25.0) | |||
DOD | 9 (25.7) | 6 (25.0) | 3 (13.0) | 7 (25.0) | 2 (28.6) | 9 (29.0) | 0 (0.0) | |||
Recurrence | 18 (51.4) | 9 (56.2) | 9 (36.0) | 0.184 | 13 (40.6) | 5 (55.5) | 0.447 | 15 (45.5) | 3 (75.0) | 0.340 |
More than 1 recurrence | 9 (25.7) | 4 (28.6) | 5 (21.7) | 1 | 5 (17.9) | 4 (57.1) | 0.294 | 7 (18.9) | 2 (50.0) | 1 |
Death patients OS (mean ± sd) | 7.0 ± 5.7 | 46.3 ± 63.9 | 0.151 | 6.0 ± 4.0 | 16.7 ± 17.8 | 0.179 | 20.1 ± 37.8 | – | * | |
Patients with recurrence DFS (mean ± sd) | 47.9 ± 63.6 | 28.0 ± 41.3 | 0.486 | 12.2 ± 11.4 | 11.4 ± 12.6 | 0.897 | 11.9 ± 12.1 | 11.6 ± 10.5 | 1 |